Monday, February 24, 2020

Week 340: Finding alignment

As of last week's post, I was feeling very optimistic. We'd had a great meeting with the new doctor, it felt like we had a treatment plan laid out, and we had a plan to alternate visits between the new doctor and the nurse practitioner so that we could have the benefit of both his expertise and her long-term connection with us. What could possibly go wrong?

Why do I even ask that question any more? Here is what is wrong, in a nutshell. It doesn't seem like our clinic has done a particularly good job, or maybe not any job at all, in terms of establishing the roles of the different providers and how they are meant to interact with each other. We have the director, the new doctor (who is sort of co-director and will become director when the current director retires) and the nurse practitioner (who mostly works with the current director and occasionally with the new co-director). So far so good, right?

Except, when I contacted the nurse practitioner to lay out what the new doctor had suggested to us, she said that she was not interested in doing the alternating visits idea and that in fact she had told the new co-director not to even offer that to patients as an option. She also said that in her opinion, the new treatment plan he proposed wasn't likely to help Lemon, but that we were welcome to try it if we wanted.

So, where does that leave us? We now have to decide if we are staying the course with the nurse practitioner (working with the current director) or jumping ship and getting care just from the new doctor. Obviously there are pros and cons to both paths. I haven't made a final decision yet, but am leaning towards switching to the new doctor. He is a relatively unknown quantity, but I had such a good feeling about him from our visit. And, I don't feel like the nurse practitioner and current director combo are really offering us a viable treatment plan to counter what the new doctor is proposing (plus, they're the ones who missed this issue when they could have caught it in June). On the other hand, I do really like the nurse practitioner and she has been working with us for a long time and knows us well.

It's a puzzle, but one I need to resolve soon so that we can stop standing still and actually do something to try to improve Lemon's situation. If I've learned one thing over the past 6.5 years, it's that nothing with CF is ever as straightforward as it should be!

Monday, February 17, 2020

Week 339: Opinions

We had a lucky break this past week. I'd asked our nurse practitioner how to go about getting a second opinion from our new CF doctor, and she sent me a message encouraging me to do it and telling me exactly what words to say to the scheduler to facilitate the scheduling of such an appointment. So, I called the scheduler, said the magic words, and...well, the concept of a second opinion had apparently never been raised with her, as such a circumstance has never before arisen at our hospital. Or something. In any case, she said she was unable to help me and would have to consult with the nurses about what to do. Suffice to say I was annoyed.

But, I was too hasty. The nurse called me back shortly thereafter, and I once again explained the situation and said the magic words, which were at least comprehensible to the nurse. Then, she started looking at the new doctor's schedule and started to say something about him being booked out quite a way. But, as she was scrolling through his schedule for May (!) another nurse came into the room and told her that the doctor had just had a cancellation that very afternoon, and could Lemon and I come in then? Yep. We sure could. I cancelled all my afternoon plans, pulled Lemon out of school, and off we went.

It was so worth it. The new doctor is fantastic. He did a good job of not actually rolling his eyes while looking at the X ray from June and hearing that no actions were taken at that time. Instead, he focused on what all the latest research says, about how important it is to intervene aggressively in kids to preserve their lung function, how FEV1 isn't an accurate measure of what's going on inside the lung, especially in kids who haven't perfected the technique yet. In summary I was very impressed by his knowledge and attitude. So, henceforth the new doctor and the nurse practitioner will be trading off visits with Lemon, but the new doctor will be at the reins, so to speak. Finally, after almost 4 years, we will have a doctor that I trust in charge of Lemon's care again.

So, in the mean time, I am just waiting to hear what our intervention plan is going to be. Most likely, a course of oral antibiotics, and probably some more increased airway clearance to help the antibiotics do their job. But, for the first time since June we will actually be doing something real to treat whatever has been going wrong since at least then. And that, my friends, feels like progress.

Monday, February 10, 2020

Week 338: On ice

On Friday of last week, Lemon didn't have school, because obviously it was Friday and why would public schools be open on Friday? In any event, I decided we should take advantage of the day off school to go to have his follow-up X-ray done. He didn't want to go, of course, but I promised that it would be quick. After all, we were going at 8 a.m., the orders were already in the computer, there would be no line, etc.

Well, we did go at 8 a.m. Were the orders already in the computer as promised? Let's see...nope. Not at all. No orders to speak of. So, the woman at the front desk had to call back to have one of the pulmonologists enter the order. But, they were busy, so she had to call a few times. After 45 minutes in the virus pit of a waiting room, we were finally taken back to X-ray. Lemon was remarkably good about the whole thing, waited very patiently and did his little wiggly best to stand still while the X-rays were taken.

After the X-rays were done, I asked the technician if I could come back and take a peek at the images on the computer screen. She said no, because there was patient information on the screen. Right, like the patient who is my child? You find yourself unable to minimize some other windows if there are any even up? No one has ever told me I couldn't see the images right away before, and let's just say this is not our first ever imaging experience. Still, by that point we mainly just wanted to leave so I didn't make a big deal about it.

Over the weekend we got the results: the collapsed area is still there. I can't say I'm too surprised. After all, it has been there since at least June, though countless sessions of the vest and multiple rounds of inhaled antibiotics. So, the hope that it could be shifted with manual PT was slight. Maybe a genuine hope, but slight.

What now? Well, the doctor had recommended that we "consider" a bronchoscopy. Of course, that seems a bit ambiguous. Is he recommending it? Or what? What I am considering at this point is a second opinion. We have a new CF doctor at our center, finally, so tomorrow I am going to schedule an appointment with him to get his opinion on how best to proceed. Hopefully he has some availability in the not too distant future. Of course I'd like to get this resolved as quickly as possible, but on the other hand if it has been lurking since June it hardly seems like waiting an extra week or two is going to make a critical difference.

Of course, this is not how we were hoping this would end up. But, on the other hand, Lemon has been doing really well these past couple of weeks (knock on wood!), gained a little weight, and is feeling fine. So, at least I feel like we are in as good a starting position as could be hoped for for whatever is coming up next.

Monday, February 3, 2020

Week 337: Close call

Today was a good reminder that everything can change in an instant. This morning, I left the house with Lemon at the usual time. We went on our usual way, heading north down our street past a couple of houses to the crosswalk. We live right next to an elementary school, so at this hour of the morning, the crosswalk is a busy place as children from the surrounding houses cross the road, heading towards a paved footpath that leads directly to the school. The crosswalk is equipped with flashing lights that are activated by someone pushing a button. There were already lots of kids about, and the button had already been pushed. A southbound driver, having seen the flashing lights and all the children, stopped his car maybe 15 feet back from the crosswalk, clearly yielding to the pedestrians, so a group of children started walking across. Lemon and I, meanwhile, had almost finished crossing the northbound side of the street, reaching the green median between the north- and south-bound sides.

Then, suddenly, a white pickup truck came down the southbound side of the street, slowed but did not stop, and struck the car that was already stopped there waiting for the children to cross. More incredibly, the truck did not stop after hitting the car, but kept going, pushing the previously stopped car closer and closer to the crosswalk until that car was practically in the crosswalk. The whole thing appeared to happen in slow motion as I was watching it, but it must have all been over in an instant. Unbelievably and thankfully, no one was injured. The stopped car had stopped far enough back from the crosswalk that even with the push from the truck, it never reached the crosswalk. The kids that were there got across, and Lemon and I watched safely from the median. The driver of the stopped car practically fell out of his vehicle, he was so shaken by the experience.

There was a police car on the scene almost immediately, joined soon after by two more police cars and a fire engine. I sent Lemon on ahead to school with another family from the neighborhood, and stayed behind to give a statement to the police. The driver of the stopped car gradually regained his composure and got back in his car, and I went back home after giving my statement. I watched from the kitchen window long enough to see the police arrest the driver of the truck and take him into custody, not before removing an open container of beer from the front of the car, draining it, and taking the container as evidence. I learned later from the police report that this was the man's 5th OWI offence.

It was incredibly lucky that no one was injured in this whole scenario. Of course I have witnessed car accidents before, and have read a thousand things about drunk driving. But this was really different. The man in the stopped car had done everything exactly right--he was driving a car in good working condition, on clear roads, on a clear day, with a clear head. He was paying attention to his surroundings, had obeyed all the laws and signals. And yet, through absolutely no fault of his own, he had almost been put in the worst situation imaginable. I have always associated images of drunk drivers with late nights, parties, bars, and the like. Never 8:20 a.m. on an unusually mild February Monday.

I'm still kind of shaken by the whole thing. Lemon had just been asking me the other day when he could start walking to school by himself. I think after today I need to revise my earlier estimate up. By at least a decade.

Monday, January 27, 2020

Week 336: Playing possum

Nothing quite gets one's blood going on a Monday morning like one's children calling excitedly "Mama! Come quick! Something furry! In the living room! I think it's a rat!" Upon further inspection, there was something furry. Most thankfully, it was not inside the living room, but rather visible through the living room window. And, it was not a rat, but an opossum. A really, really huge opossum. We watched it, it watched us. And then we went about our respective days.

Other than that, I am thankful to report that it has been a fairly uneventful week out here on the frozen tundra. We have continued along with our manual chest PT regimen, and have another two weeks or so remaining before the repeat chest X-ray. We are just fervently hoping that Lemon does not catch something else in the mean time. The viruses are flying fast and furious in Wisconsin at the moment, so much so that the children's hospital took the precaution of banning all visitors under the age of 12 from the inpatient floors. Not exactly a comforting feeling. But, the kids still have to go to school and such so we just try to emphasize good hygiene and hope for the best. Well, that and I handed Lemon's teacher a giant 4-pack of sanitizing wipes this morning before school. We'll refer to that as active hoping.

I got Lemon on the scale again this evening just to see where things are and he seems to be holding steady for now. Still not quite as heavy as he was at the end of August, but no longer as light as he was right after all the recent illnesses. I would love it if we could get a little bit more weight back on him, but honestly just holding steady is a pretty good accomplishment for the winter months. And, to be fair, we are holding steady at something like the 75th percentile for BMI so really not too shabby.

I'm feeling good that January is almost over, so only another couple of months of winter to go. Amazingly, of course, that means the complex coordination of summer plans has already begun, complete with signing up for camp, planning of vacations, etc. Hard to believe that in less than 6 months we will be back on the beach, and Lime will turn 5. Things do seem to move right along!

Monday, January 20, 2020

Week 335: Tenure

As you all know, I do try to keep this blog by and large about the little citrus folks, but every once in a while we larger people can intrude on the narrative. And, I feel like if there were ever a week that such an intrusion would be justified, it would be this week, where I can share the great news that Papa Bear is now officially a tenured professor! He worked incredibly hard for this and it was well-deserved (and a surprise to no one) but nonetheless a tremendous accomplishment and we are all very proud of him.

Of course, on the morning of the tenure announcement, where were Lemon and I? At Children's hospital, of course. Where else would we be, really? We had our annual follow-up with GI. As always, more questions than answers, but here is what we know. Lemon's liver ultrasound has findings that, in an adult, would be consistent with "mild fibrosis." Because the technology is so new, there is no accepted pediatric standard for the measurements. So, no one knows how to interpret it really. But, between that and the bloodwork the doctor believes that everything is normal-ish, for a kid with CF. So, we were told to basically stay the course, continue on our current dose of ursodiol, and repeat the ultrasound this coming summer, along with a repeat of the bloodwork. I am going to take the whole thing as a case of "no news is good news" and move along.

We have been continuing with our daily regimen of manual chest PT in addition to the vest in an effort to get the collapsed area of Lemon's lung to reinflate. I really wish we had some interim way to tell if it was doing anything. Or some way for me to know if I am doing the PT in the best possible spot. But, of course, no and no. So, we are doing our best and we will see where things stand next month. I am trying to line up a second doctor to look at the x-ray and give us an opinion on how to best move forward based on what we see there, so stay tuned on that front.

Other than that, we are just keeping our fingers crossed that we can avoid illnesses for a few weeks at least, so Lemon can get some more weight back on and so forth. Not going to school certainly helps. I feel as though there were just two weeks of vacation, and yet somehow Lemon only has school 3 days this week. I know, I know, I have turned into a curmudgeon. But I came by it honestly.

Monday, January 13, 2020

Week 334: Seen in June

Nothing like a little CF drama to start off a new year. So, as you loyal readers will recall, about a month or so ago Lemon had a chest CT as part of a research study that he is in. And, that, to my disappointment, the CT itself would not be shared with our clinical team, but rather that they would just be notified in the event of "significant findings." Well, guess what, there were significant findings. 
The significant finding is RML atelectasis, or an area of the right middle lobe of Lemon's lung that has collapsed. This is, of course, not great news. The recommended course of action is to do a full month of manual chest PT (focused on the right side) in addition to our regular airway clearance regimen to try to clear the collapsed area and get it to reinflate. We will get a chest X-ray after the month of extra therapy to see if that has happened. If the area does not reinflate, it may be that we will need to have a bronchoscopy to try and reinflate it. 

None of that is ideal, of course. What is far less ideal is that, after receiving the report from the research CT, the doctor who is the head of our CF center went back to the last X-ray that Lemon had done (IN JUNE, MIND YOU) and said that actually, he could see evidence of the area of collapsed lung there, too. This does seem to beg the question of what we are doing the annual X-rays for, if not to catch things like this early, when we have the best chance of intervening before lasting damage is done. As of now, the doctor estimated that we have a 50:50 shot at getting the area to reinflate. He also said something about how good it was that we caught it early. Early? We could have caught it 6 months ago, apparently, and we wouldn't have caught it until 6 months from now at our next scheduled X-ray (if then!) if I hadn't decided to sign Lemon up for that CT. So I hardly view this as an occasion for self-congratulation.

Suffice to say I am very disappointed by how this has all unfolded, and am definitely planning on getting an additional opinion once the next chest x-ray is done. Luckily a new pediatric pulmonologist has joined our CF center in the interim, so I will start there.

Meanwhile, we are following up with the GI doctor on Friday of this week. Now, of course, I am really wondering about the conflicting reports that I got on the results of our last liver ultrasound, which were either "normal" or "mild fibrosis" depending on who you ask. At this point, I am very interested to hear directly from the GI doctor.  

So, not really the start to 2020 that we were hoping for, but at least Lemon is feeling fine and has managed to gain back some of the weight he lost in December, so we are in a better position for whatever lies ahead.